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#PCNSL
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#CNSTo200M
#NCA
1 NHL a week Series.
Let us have a small summary / #tweetorial on Primary CNS Lymphomas

PCNSL - confined to brain, leptomeninges,eyes,spinal cord.
~ 2% of primary CNS tumors.
median age-65y
mc presentation: Focal neurologic deficits
Imaging choice : Contrast-enh MRI
Predictors of poor prognosis:age>60 y,ECOG>1, LDH,CSF protein ,inv of deep areas

PATHOLOGY (1):
90% –DLBCL
Rest :T-NHL, poorly characterized low-grade NHL, or Burkitt's
> 90% PCNS DLBCL-ABC subtype
NEWER INSIGHTS :The B-cell receptor (BCR) signaling axis with its downstream target,NFkB, is affected by mutations in MYD88, CD79B &rarely CARD11.

PATHOLOGY CONT(2)
notable risk factor - immunodeficiency
HIV infection, iatrogenic immune suppression, and congenital immune deficiency
PCNSL may be a consequence of EBV-mediated clonal expansion and malignant transformation of B-lymphocytes

PATHOLOGY CONT (3)
Most PCNSL cells demonstrate IgHV genes that have high levels of somatic mutations and show intraclonal heterogeneity pointing towards their derivation from mutated germinal center B cells
Here is a bonus tweet of pathology in PCNSL https://twitter.com/anjuthevirgo/status/1293285265964822528?s=20

MANAGEMENT :
Rx: IV HD-MTX at var doses (1–8 g/m2) with other chemotherapeutic agents and/or WBRT
-no consensus on the optimal dose of MTX/on the role of radiation
-Rituximab is incorporated in these regimes.
Here is an awesome yet simplified approach @UpToDate based on age

NOVEL Insights :Modulators of BCR pathway:
BTK inhibitor-ibrutinib
IMiDs like lenalidomide because of inhibition of NF-κB activity+PI3K/AKT pathway
in relapsed refractory cases is promising
Observation of altered PD-L1gene has led to trials of Nivolumab in PCNSL

NOTEWORTHY POINTS:
If possible,biopsy shud b done before steroids
Therapy-high dose MTX [email protected]/m2
Despite inadequate data supporting rituximab, excellent tolerability+good results in prospective trials, RTX may be favored IN front line RX
DONOT FORGET TESTIS

COMMONLY USED PROTOCOLS
We prefer using the R-MPV protocol in our centre.

kindly add on to this dear seniors @DrGPrakash @IJMPOofficial @bagalbp @DrPMPGI @rahulbhargavadr @BollamRajesh4 @pb10_bmt @blood_academy @prantar @EshaKaul1 @dixit_hemat @HariMenon68 @ReetuJain19 @drkunalsehgal @satya_yadav @DimPositive